Eosinophilic lung diseases
Chai Gin Tsen Department of Respiratory and Critical Care Medicine
Tan Tock Seng Hospital The eyes do not see what the mind does not know
Not very common
A high index of suspicion is required Introduction
• Increased eosinophils in one or more compartments of the lung – airway, pleural, parenchyma. • Lung involvement + peripheral eosinophilia = Eosinophilic lung disease ?
• Eosinophilic lung diseases may not always have peripheral eosinophilia. • Not all patients with peripheral eosinophilia has eosinophilic lung disease The obligatory classification slide
• Eosinophilic (Eos) lung disease of undetermined cause • Lung limited: acute eos pneumonia, chronic eos pneumonia • Systemic: Eos granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), hypereosinophilic syndrome
• Eosinophilic lung disease of determined cause (secondary causes) • Parasites • Allergic bronchopulmonary aspergillosis (ABPA) • Drugs and toxins • Others: malignancy, asthma, idiopathic interstitial pneumonia (IPF, chronic HP, Cryptogenic organizing pneumonia) Hence history should include
• Symptoms: Acute/subacute/chronic • Occupation/environmental exposures • Drugs • Travel history • Infectious symptoms • History of asthma • Systemic symptoms Investigations
• Directed by history • May include, but not limited to: • Lung function tests • ANCA • Serum IgE levels • Stool for ova cyst parasites • Bronchoscopy and bronchoalveolar lavage
• May need ID, hematology or rheumatology consult My approach Peripheral eosinophilia
1. If it is persistently elevated, evaluate for the causes of eosinophilia – long list of causes 2. Is there pulmonary involvement? 3. Are lungs the only organ involved? Or part of a systemic disease? 4. Evaluate for secondary causes of pulmonary eosinophilia 5. Primary causes – diagnosis of exclusion Other causes of peripheral eosinophilia
• Allergies – food, drug, environment • Worms and other infectious disease • Malignancy especially hematological malignancy • Other organ involvement can also cause peripheral eosinophilia eg GI, renal, dermatology etc. Case 1 Case 1
• 45 year old Indian male • Cough x 3/52 with fever for 1/52 • Symptoms not better after a course of clarithromycin • Recently returned from India • Referred from ED to TBCU for possible PTB
Progress
• Following tests were ordered : • Sputum AFB smears • Mantoux test • Another course of antibiotics • Review in 1/52
• Then we received a call from Dr Cynthia Chee
How come you did not see the eosinophil counts done in ED? Progress
• Treated as tropical pulmonary eosinophilia • Diethylcarbamazine x 2/52
• Not surprisingly AFB cultures were negative
Tropical pulmonary eosinophilia
• Associated with mosquito-borne filarial parasites Wuchereria bancrofti and Brugia malayi. • Endemic in Indian subcontinent, SEA, S America, Africa, S Pacific islands. • Immune hyper-responsiveness to microfilariae trapped in the lungs. • Midnight blood film to “catch” the microfilariae in blood. • Serologies • Treatment: Diethylcarbamazine for 2 to 3 weeks • 20% may relapse Simple pulmonary eosinophilia (Löffler syndrome) • Usually more acute • Transient, mild self limited hypersensitivity response to parasites migrating through the lungs. • Migratory pulmonary infiltrates • Ascaris lumbricoides, Strongyloides stercoralis etc • Treatment: Mebendazole Case 2 Case 2
• 54 year old Indian male • Previous history of PTB in 2003 complicated by bronchiectasis • Worsening cough, greenish sputum, dyspnoea over 6 months • On and off fever
Differential diagnosis of “fleeting” or “migratory” pulmonary infiltrates on CXR
• Aspiration pneumonia • Drug induced lung injury • Cryptogenic organizing pneumonia • Simple pulmonary eosinophilia • Chronic eosinophilic pneumonia • Allergic bronchopulmonary aspergillosis
Proximal bronchiectasis
Mucus plugging
Tree in bud opacities
Peribronchial thickening Progress
• Broncho-alveolar lavage • Bacterial, AFB cultures NEG • Fungal culture: Aspergillus flavus
• Total IgE > 1000 • Aspergillus antibody NEG Started on Prednisolone and Itraconazole
Allergic bronchopulmonary aspergillosis (ABPA) ABPA
• Not very common: • Only complicates 1-2% of asthmatics and 2-15% with cystic fibrosis (CF) • Rarely it can happen in patients without asthma or CF • Abnormal host response to fungus • Consider in poorly controlled asthma • Treatment: steroids ± itraconazole Case 3 Case 3
• 72 year old Chinese man • Admitted for non-resolving pneumonia – persistent lung infiltrates despite multiple courses of antibiotics • Ex smoker • Had been taking TCM • Rhonchi heard on physical exam Mar 18 Feb 18 Investigations
• CRP 19.4 • Sputum AFB smear x 2 NEG
CT thorax Further investigations
• Stool ova cyst parasites: NEG • ANCA NEG • BAL ova cyst parasites NEG Diagnosis: Chronic eosinophilic pneumonia Prednisolone started Mar 18 May 18 Chronic eosinophilic pneumonia
• Chronic onset dyspnoea, cough, wheeze • < 10% has no peripheral eosinophilia • BAL eosinophilia • Treatment: Corticosteroids – taper over 6 to 12 months • Relapse up to 50% The classical photographic negative of pulmonary oedema
Only seen in <25% of the cases Acute eosinophilic pneumonia
• Presentation similar to ARDS, severe community acquired pneumonia or acute interstitial pneumonia • Smokers • 2/3 need mechanical ventilation • Peripheral blood eosinophilia not prominent • BAL eosinophilia • High dose steroids with rapid improvement Case 4 Case 4
• 71 year old Chinese man • Non smoker • History of stiff person syndrome on long term prednisolone • Cough for 2 weeks, fever, hemoptysis, hypotensive, hypoxic. In this instance, there is no eosinophilia 8 Dec 2016 15 Dec 2016
DESPITE IV PIP/TAZO + VANCOMYCIN Progress
• Patient was deemed too sick for bronchoscopy and BAL (on 50% ventimask) • Bacterial cultures, AFB smears, PCP from induced sputum were all negative Then this happened
• An astute microbiology lab technician saw larvae on the sputum gram stain. • Bronchoalveolar lavage done one week later when patient improved: • Cell counts – unable to perform • Strongyloides not seen in BAL • Bacterial cultures: E Coli • PCP NEG • AFB cultures NEG • Fungal cultures yeast 1+ Progress
• Covered with Ivermectin and albendazole • Iv ceftriaxone for E Coli CXR 6 months post discharge Strongyloides hyperinfection
• Immunocompromised host • Heavy hematogenous seeding to various organs including lungs, liver, heart, CNS. • 30-45%: Gram negative septicemia from GI source • High mortality • Optimal treatment duration uncertain – determined by clinical response Hong Kong case series
None of the patients had peripheral eosinophilia.
Could be due to long term immunosuppression or gram negative septicemia
Lam CS et al. Eur J Clin Microbiol Infect Dis 2006 In summary
• Know the causes of peripheral eosinophilia • Eosinophilic lung disease can occur with or without systemic involvement • If there is eos + lung involvement: evaluate for secondary causes first • Some cases of eosinophilic lung disease do not have peripheral eosinophilia: • Strongyloides hyperinfection • Acute eosinophilic pneumonia • Chronic eosinophilic pneumonia (<10% of patients)
• Don’t forget the other causes: malignancy, asthma, idiopathic interstitial pneumonia (IPF, chronic HP, Cryptogenic organizing pneumonia)
Thank you